Case report of a 52-year-old woman with angiomyolipoma of the left kidney. The tumor had appearance of a typical angiomyolipoma predominantly composed of spindle cells, some epithelioid cells and few large hyalinized vessels. Adipose tissue was concentrated into small foci. Unusual presence of large cells with a clear fine granular cytoplasm closely resembled cells of the "sugar tumor" of the lung. Epithelioid cells and occasionally spindle cells were HMB45 positive. A minority of cells also coexpressed S100 protein. Clear cells were usually strongly positive for HMB45 too. Our findings supported consideration of a close relation between clear cell ("sugar") tumor of the lung and angiomyolipoma.